the nephrotic syndrome def: it is a clinico -biochemical state of many causes features 1-heavy...
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The nephrotic syndrome
Def: It is a clinico -biochemical state of many causes
Features1-Heavy proteinuria.2-Hypoproteinemia.( decrease protein in the blood)3-Generalized oedema.4-Hyperlipidemia and lipiduria
Causes
Renal1-Membranous GN2-membranoproliferative GN3-Minimal change GN4-Focal segmental GS5-Focal GN.(Mesangial,IgANephropathy)
Systemic Diseases1-SLE2-DM3-Amyloidosis4-Infections e.g.; malaria, HBV,BSyphilis6-lymphoma7-Drugs:gold salt and NSAI
Common features of nephrotic syndrome
Gross
-Enlarged pale kidney. -yellow ting due to fat resorption by tubular epithelium.
Microscopic
1-Glomeruli;• LM&IF: Features specific to the disease.• EM: Fusion of foot processes of podocytes.
2-Tubules• 1-Hyaline droplets. • 2-Vacuolar degeneration due to resorption of
fat • 3-Hyaline casts
3-Interstitial tissue
variable oedema
Membranous GN
Age: adults (30-50 ys)
Cause:--Primary :Unknown (85%)-Secondary in course of infection like malaria syphilis, HBV, B, malignant tumors and gold salt therapy
Patho: ICD
Light:Thickening of the GBM
IFGranular flourescences to IgG and C3
EM: -Fusion of foot processes Of podocytes.-Subepithelial deposits
Microscopic
EM in MGN, the darker electron dense immune deposits are seen scattered within the thickened
basement membrane.
Clinical and laboratory findings Nephrotic syndrome
Prognosis: Remission and exacerbation, finally chronic renal failure.
Minimal change GNAge: Commonest cause of nephrotic syndrome in children (1-4 ys )
Pathog: unknown or it is a disorder of T cells cytokines that cause loss of epithelial foot processes
Gross: as Nephrotic syndrome.
Msc:1-Light
Glomeruli ; no changes.
Tubules and interstitial tissues show changes of nephrotic syndrome
2-IF: Negative.3-EM: Fusion of foot processes of podocytes
Clinical and laboratory findings : as NS with selective proteinuria.
Prognosis: good response to steroid therapy
Membranoproliferative GN
Age; any age, mainly late childhood
Pathogenesis;
Type I; Common. It is ICD
Type II (Dense deposit disease) :rare mediated by activation of the alternative complement pathway.
Hypercellularity
Accentuated lobulation
Patchy irregular thickening of GBM
Double contour of
GBM
MSC;1-Light
This silver stain demonstrates a double contour to many basement membranes, or the "tram-tracking" that is
characteristic of MPGN
Clinical and laboratory findingsPresentation
Nephrotic syndome+ hypertension
Nephritic syndrome
Asymptomatic proteinuria
Prognosis : remission exacerbation and finally chronic renal failure
Focal segmental glomerulosclerosis
Microscopic: -Sclerotic segments in some gl. and Hyalinosis -tubular atrophy -interstitial fibrosis
IF: Granular fluorescence of the GBM for IgM andC3.EM:Fusion of foot processes and detachement of epithelial cells
Clinically: Nephrotic syndrome, may be hypertension and microscopic hematuria.
Prognosis: unfavorable (ending in chronic renal failure
Cause: -idiopathic -In association of SBE,SLE, Henoch-schonlein PAN, and Goodpasture’s syndromePathogenesis: -ICD -Activation of the alternative complement pathway by aggregation of IgA.(Berger’s disease)
Focal glomerulonephritis
IgA Nephropathy (Berger’s Disease)
-Common in children and young adults- Recurrent hematuria-It follows infection of the respiratory,GI
and urinary tracts.-The IgA is deposited mainly in
mesangium, which then increases mesangial cellularity
MSC: Focal and segmental proliferation of mesangial cells+ necrosis and crescent formation
Clinically: Hematuria, proteinuria and may be nephrotic syndrome
Course: Subsides without residual renal impairment
Chronic GNDef: it is end stage renal glomerular disease.
Grossly:-Small contracted kidney.-Granular outer surface.-Firmly adherent capsule.-Loss of differentiation bet. cortex and medulla.-Thick BVs at corticomedullary junction.
Msc:
Glomeruli: -Hyalinised and sclerotic.-Some are hypertrophied.
Tubules are atrophied and
dilated
Interstitial fibrosis and
chronic inflammatory cell
infiltration
Thick walle-blood vessels end arteritis obliterans
Clinical and laboratory Findings:
Marked hypertension
IncreaseBl. urea
Urine changes-Polyuria.- low Specific gra.-Mild albuminuria.-Hyaline and -granular casts
Prognosis: without Treatment is poor
Small- Sized Kidney (contracted kidney)
1-Hypoplastic kidney.
2-Chronic GN
3-Chronic PN
4-Senile(atherosclerotic) kidney.
5-Kidney of benign hypertension (Benign nephrosclerosis).
DMEffects of DM on the kidney:-Diabetic GS-Renal arteriolar sclerosis.-pyelonephritis.-papillary necrosis.
Diabetic GSIt leads to:a-Proteinuria.B-Nephrotic syndrome.C-CRF.
MSC: 1-Diffuse GS.-Diffuse increase in mesangial matrix-Thickening of GBM2-Nodular GS. (kimmelsteil Wilson disease)Hyaline nodule is present in the mesangium,Containing fibrin and lipid.
3-Insudative lesion:-fibrin cap; eosinophilic focal Thickening of peripheral capillary loop.-Capsular drop: eosinophilic thickening of Bowman’s capsule
Lupus nephritis
Presentation: Recurrent hematuria,nephritic s,nephrotic s,hypertension,CRF.
Classification;-class I:Normal kidney.-Class II:Mesangial glomerular lesion.-Class III:Focal proliferaive GN.-Class IV:Diffuse Proliferative GN.-Class V:Membranous GN.-Class VI:Advancing sclerosing GN.
MSC of Class IV: Diffuse Proliferative GN
-Diffuse hypercellularity due to Proliferation of endothelial cells and mesangial cells -Irregular thickening of GBM - Wire loop appearance-Few epith.crescents-Hematoxylin bodies.
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